Vadehra Deepak, Davino Tammy, Datta Debapriya
Internal Medicine, University of Connecticut Health Center, Farmington, USA.
Critical Care, University of Connecticut Health Center, Farmington, USA.
Cureus. 2020 Apr 21;12(4):e7769. doi: 10.7759/cureus.7769.
Acute chest syndrome (ACS), a vaso-occlusive crisis in patients with sickle cell anemia, is a life-threatening condition and a leading cause of death in these patients. It is treated with analgesics, antibiotics, intravenous fluid, supplemental oxygen (or ventilatory support in severe cases) with simple or exchange transfusion, being the mainstay of therapy. We report a young Jehovah's Witness (JW) patient with sickle cell anemia who presented with ACS. Her religious beliefs precluded the use of blood products. Despite concomitant hemolytic and aplastic crisis and a hemoglobin nadir of 3.1 g/dL, the patient was treated successfully with supportive care - including mechanical ventilation, sedation, paralysis, and erythropoiesis stimulation - and survived. A maximal supportive strategy consisting of ventilatory support with a high fraction of inspired oxygen, sedation, paralysis, erythropoiesis stimulation, and limitation of blood draws can result in the successful treatment of JW patients who refuse blood products.
急性胸部综合征(ACS)是镰状细胞贫血患者的一种血管闭塞性危象,是一种危及生命的病症,也是这些患者死亡的主要原因。其治疗方法包括使用镇痛药、抗生素、静脉输液、补充氧气(严重病例采用通气支持)以及简单或换血输血,这是主要的治疗手段。我们报告了一名患有镰状细胞贫血的年轻耶和华见证会(JW)患者,该患者出现了急性胸部综合征。她的宗教信仰使她不能使用血液制品。尽管同时出现了溶血性和再生障碍性危象,血红蛋白最低点为3.1g/dL,但该患者通过支持性治疗(包括机械通气、镇静、麻痹和促红细胞生成刺激)成功治愈并存活。由高浓度吸氧通气支持、镇静、麻痹、促红细胞生成刺激和限制抽血组成的最大支持策略可以成功治疗拒绝使用血液制品的JW患者。